Tuesday, 31 January 2012

Kalydeco (VX770) approved by FDA

Super excited for those eligible in the states!
Let's hope cost isn't too much of an issue.

Fantastic news for the CF community. Will blog in depth when I get chance.

Link 1

link 2

Press release
Let's hope this is the start of great things for all.


Monday, 23 January 2012

The morning after the night before.


You guessed it, that's me.

Having hope is not a bad thing and it is hope that drives you on in life. Without hope, you would be a glass is half empty kind of person and we all know that isn't a fun way to live.

For over two weeks I have been clinging on to hope and as each day passed, I began to breathe a sigh of relief that maybe, just maybe this time things were going to be different.

Slightly misguided and lulled into a false sense of security allow me to indulge you all in our weekend of full blown rage. I'm not talking about any old type of rage, I mean Cipro rage.

The beast returned with a vengeance.

Sophie has been a little grumpy but no more than usual tiredness, we didn't think any more of it as most of the time she was super pleasant.

Cue Sunday, started off okay, visited her nan and grandad and all seemed okay, temper a little more short than usual but coping.
Getting ready to leave and temper more frayed, tears and generally irritable now.

By the time we arrived home, Sophie had morphed into a Cipro induced beast.
Throwing things, kicking, punching and head butting stuff, after coaxing her into her treatments and getting her bathed and ready for bed she just flipped.

Somehow she managed to find the strength to pull her mattress off her bed. (I struggle to move this) and she just continued to throw her stuff about, attacked her toys, attacked her wall and anyone who went near her. She trashed her room.

It was awful and took a very long time for her to calm down and fall off to sleep. I text her CF nurse and asked if the Cipro could be stopped as we were nearly at the three week mark any way.

I had a pit of fear in my tummy every time I climbed the stairs of what I was going to find. If it was physically possible her head would have spun last night. Everything about her had changed including her facial expressions, she was so agitated.

I just cannot imagine what thoughts were running through her head to make her go from being so friendly and lovable to completely violent and vile in such a short space of time.

I have gained even more respect for parents who deal with challenging behaviour on a daily basis. I just don't know how you do it both physically and mentally as I feel absolutely exhausted today.

Sophie's CF nurse has text me back and the Cipro is now stopped. Just hope it gets out of her system quickly!!

In other news.

CF clinic went really well last week, no issues at all which is brilliant. Her consultant would like her to have a CT scan of her lungs so we have a baseline picture in detail of what her lungs actually look like.

Although Xrays are useful, they don't show the finer details of the small airways which is typically where lung damage first occurs. In these areas, a change in health is not usually noticed either and it's this silent side of CF that scares me a little. For now we wait for the appointment and hope we can get Sophie to cooperate.

One last piece of news, Sophs sputum culture from clinic is back...

Mixed Growth.

Yep, completely normal and although I know pseudo will show again soon, it's nice to know the numbers are so low it's retreated and is in hiding for now at least.

Off to hospital for Sophs port flush tomorrow, I just hope the Cipro is leaving her system by then otherwise it may get ugly!

Take care and thank you so so much for all of the lovely messages and comments. They mean a lot to us :)


Tuesday, 17 January 2012

Huge catch up!

Well hello blogging world.

I thought I'd better update the last couple of months and apologies in advance there are lots of pictures. You know how I like to use visual aids to get my point across :)

The picture above haunts me terribly but I have included it to emphasise just how poorly Soph has been and how obvious it is to know when she is ill.

Lets rewind.

Soph had completed a two week course of clarithromycin for the new bug she had cultured-strep pneumoniae, but unfortunately she didn't improve at all. In fact, she was much worse, so after speaking to her team again, it was decided to continue with a further two week course of clarithromycin as Christmas was approaching fast.

After four or five days, Soph was continually getting worse, becoming lethargic and grumpy, coughing and wheezing throughout the night so I called the team again. They felt that the wheeze was probably due to her airways being inflamed from all of the infection she was shifting. At this point her sputum was thick and green.

So added to her daily treatment regime was prednisolone (steroid) and a salbutamol inhaler to be used via a spacer.

Knowing Soph and how she reacts to new treatments we actually never expected her to use the inhaler. Her CF team just said as long as we could get some into her it would be better than nothing and the plan B would be for her to have it via a nebuliser instead. We gave the inhaler a try as I didn't fancy adding a 5th nebuliser into the day unless absolutely necessary.

To our surprise, Soph loved it!! I guess she is my daughter, which makes her an oddball too :D

Over the next week, Soph appeared to be turning a corner and was still at school with an irritating cough only. We thought the meds were kicking in and we would soon see the back of this run of illness.


Two days before Soph broke up for the holidays the school rang to say Sophie really wasn't well and wanted me. When I got to school she looked awful, super pale and could barely walk or speak.
I scooped her into the car and within minutes she was asleep. The very first photo in this blog was taken 10 minutes after I picked her up from school.

Scary times.

For the next few days, Soph remained the same and Ian and I even had to pick her up and put her on the toilet. She just lay on the floor or sofa and slept. Then the temperature appeared at 39.9C and we battled to keep it at 38C with calpol for a few days.

Soph then began to develop tummy ache and retching. Now at this point we were not sure if she had a stomach virus or a partial blockage as she cannot be sick due to her nissen fundo.

We just rode it out until she started having her bowels open-mustard yellow if you're curious and don't worry, no picture of that!

So the assumption is, she had picked up some sort of gastric viral bug on top of her chest infection and her poor little body just shut down and went into sleep mode.

Finally she turned a corner just after Christmas and two days before she was due to start back at school she was finally her old self again.
Clarithromycin finished and we sent in another sputum sample to make sure the strep had truly gone.

5 days of having no form of antibiotics we got a phone call to say Soph had grown pseudo again but it was a pathetic growth and her CF consultant wanted to try one last ditch attempt at eradication.

This I found incredibly strange as he has always said she was colonised but we went with it, nothing to lose. So 3 weeks of ciprofloxacin was started.

Yes the drug that has sent her psychotic in the past.

However this time round, she has been an absolute pleasure so maybe it was her age that caused such a severe response several years ago?

AND this brings us to present day.

Soph has approx 1 week left of cipro to go and when this course is complete, she will have had 9 straight weeks (minus the 5 day break) of some form of antibiotics.

The antibiotic train comprised of:

Two weeks of IVs, 4 weeks of clarithromycin and 3 weeks of cipro, it's insane, but couldn't have been helped due to how poorly she was. There was talk of bringing her into the hospital for more IVs if she didn't improve.

Thank goodness she rallied.

So after the mammoth catch up, I will wish you all a very happy new year and leave you with a few more pics of Soph looking like her old self again!

This was not long before she became really poorly, she had been to a fancy dress party and was snow white.

Taken today on the way home from CF clinic,

Playing in the frost, she loves getting fresh air and I have to drag her in when it is so cold.

I will update about CF clinic over the next week as I feel you've had enough to read for one sitting!

Take care all and thank you so much for all of the well wishes and support, we couldn't get through it without you all.

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